Abstract：
OBJECTIVE: Olfactory dysfunction (OD) commonly occurs in patients with sinonasal dysfunction, but the prevalence and severity of olfactory issues in adolescents with cystic fibrosis (AwCF) is unclear. OD may contribute to dietary deficiencies and exacerbate nutritional challenges. We sought to review literature on the effectiveness of medical and surgical management of sinonasal symptoms in AwCF and the associated impact on olfactory function.
METHODS: We performed a systematic literature search of PubMed, Embase, Web of Science, and Ebsco CINAHL from 1980 to 2022 per PRISMA-ScR protocols to conduct a scoping review in an effort to compile data on study design, patient demographics, clinical characteristics and outcomes, along with risk of bias.
RESULTS: Of 368 abstracts, 3 articles exclusively evaluated AwCF for a total of 34 patients. Two studies evaluated endoscopic sinus surgery (ESS) and dornase alfa. An additional 6 articles were included for mixed pediatric and adult CF populations totaling 313 patients. Interventions included ESS, elexacaftor-tezacaftor-ivacaftor (ETI), ivacaftor, saline, dornase alfa, hyaluronic acid, and hyaluronic acid-tobramycin combination. Outcome measures included subjective assessment of OD using non-validated (4/9) and validated (4/9) surveys, and psychophysical (1/9) smell testing. Studies evaluating ESS, FESS, dornase alfa, ivacaftor, and both hypertonic and isotonic saline reported statistically significant improvement in OD, whereas ETI failed to improve OD despite improvement in other quality of life measures.
CONCLUSIONS: There is limited data regarding the impact of medical and surgical interventions on olfaction for AwCF. Assessment of olfaction was often limited to subjective and qualitative self-report. We suggest that tracking of olfactory outcomes with psychophysical testing is critical in this population with dietary challenges and weight management issues.
METHODS: We performed a systematic literature search of PubMed, Embase, Web of Science, and Ebsco CINAHL from 1980 to 2022 per PRISMA-ScR protocols to conduct a scoping review in an effort to compile data on study design, patient demographics, clinical characteristics and outcomes, along with risk of bias.
RESULTS: Of 368 abstracts, 3 articles exclusively evaluated AwCF for a total of 34 patients. Two studies evaluated endoscopic sinus surgery (ESS) and dornase alfa. An additional 6 articles were included for mixed pediatric and adult CF populations totaling 313 patients. Interventions included ESS, elexacaftor-tezacaftor-ivacaftor (ETI), ivacaftor, saline, dornase alfa, hyaluronic acid, and hyaluronic acid-tobramycin combination. Outcome measures included subjective assessment of OD using non-validated (4/9) and validated (4/9) surveys, and psychophysical (1/9) smell testing. Studies evaluating ESS, FESS, dornase alfa, ivacaftor, and both hypertonic and isotonic saline reported statistically significant improvement in OD, whereas ETI failed to improve OD despite improvement in other quality of life measures.
CONCLUSIONS: There is limited data regarding the impact of medical and surgical interventions on olfaction for AwCF. Assessment of olfaction was often limited to subjective and qualitative self-report. We suggest that tracking of olfactory outcomes with psychophysical testing is critical in this population with dietary challenges and weight management issues.
摘要：
目的：嗅觉功能障碍（OD）常见于鼻窦功能障碍患者，但青少年囊性纤维化(AwCF)患者嗅觉问题的患病率和严重程度尚不清楚.OD可能导致饮食不足并加剧营养挑战。我们试图回顾有关AwCF鼻窦症状的药物和手术治疗的有效性以及对嗅觉功能的相关影响的文献。
方法：我们对PubMed进行了系统的文献检索，Embase,WebofScience,和EbscoCINAHL从1980年到2022年根据PRISMA-ScR协议进行范围审查，以努力编制研究设计数据，患者人口统计学，临床特征和结果，还有偏见的风险。
结果：在368篇摘要中，3篇文章专门评估了总共34名患者的AwCF。两项研究评估了内窥镜鼻窦手术（ESS）和dornasealfa。另外6篇文章纳入了儿童和成人CF混合人群，共313例患者。干预措施包括ESS，elexacaftor-tezacaftor-ivacaftor(ETI)，ivacaftor,盐水,dornasealfa,透明质酸，和透明质酸-妥布霉素组合。结果指标包括使用未经验证（4/9）和经过验证（4/9）的调查对OD进行主观评估，和心理物理(1/9)气味测试。评估ESS的研究，FESS,dornasealfa,ivacaftor,高渗和等渗盐水都报告了OD的统计学显着改善，尽管其他生活质量指标有所改善，但ETI未能改善OD。
结论：关于药物和手术干预对AwCF嗅觉影响的数据有限。嗅觉评估通常仅限于主观和定性的自我报告。我们建议在有饮食挑战和体重管理问题的人群中，通过心理物理测试跟踪嗅觉结果至关重要。
方法：我们对PubMed进行了系统的文献检索，Embase,WebofScience,和EbscoCINAHL从1980年到2022年根据PRISMA-ScR协议进行范围审查，以努力编制研究设计数据，患者人口统计学，临床特征和结果，还有偏见的风险。
结果：在368篇摘要中，3篇文章专门评估了总共34名患者的AwCF。两项研究评估了内窥镜鼻窦手术（ESS）和dornasealfa。另外6篇文章纳入了儿童和成人CF混合人群，共313例患者。干预措施包括ESS，elexacaftor-tezacaftor-ivacaftor(ETI)，ivacaftor,盐水,dornasealfa,透明质酸，和透明质酸-妥布霉素组合。结果指标包括使用未经验证（4/9）和经过验证（4/9）的调查对OD进行主观评估，和心理物理(1/9)气味测试。评估ESS的研究，FESS,dornasealfa,ivacaftor,高渗和等渗盐水都报告了OD的统计学显着改善，尽管其他生活质量指标有所改善，但ETI未能改善OD。
结论：关于药物和手术干预对AwCF嗅觉影响的数据有限。嗅觉评估通常仅限于主观和定性的自我报告。我们建议在有饮食挑战和体重管理问题的人群中，通过心理物理测试跟踪嗅觉结果至关重要。
